Optimal stimulation of the thalamic centromedian nucleus in children with Lennox-Gastaut syndrome: patient series

Abstract

Background: Lennox-Gastaut syndrome (LGS) is a severe developmental and epileptic encephalopathy characterized by multiple seizure types, intellectual disability, and distinctive EEG findings. Deep brain stimulation of the centromedian nucleus (CM-DBS) is an emerging therapy for LGS, but pediatric experience remains limited.

Observations: The authors report a single-center experience with CM-DBS in 6 children and adolescents (ages 12-18 years) with LGS. One patient experienced infection-related device removal after 2 months. The remaining 5 received sustained stimulation for 2.5-5 years. All 5 showed caregiver-reported improvements in seizure burden and alertness, with corroborating Clinical Global Impression-Improvement scores. Three patients exhibited marked seizure reduction following targeted reprogramming toward a previously identified optimal target of stimulation in the anterolateral CM. Pre- and post-DBS scalp EEG recordings were available in 1 patient and showed a reduced burden of interictal discharges.

Lessons: CM-DBS is a promising treatment for pediatric, medically refractory LGS when resective approaches are unsuitable. Benefits were sustained over years. Lead localization and direction of stimulation appear important to optimize clinical benefit. These findings support the feasibility and safety of pediatric CM-DBS and highlight the need for prospective trials incorporating EEG-based outcomes and patient-centered measures including comorbidities and quality of life. Early, network-targeted neuromodulation may improve long-term outcomes. https://thejns.org/doi/10.3171/CASE25478.

Keywords: Lennox-Gastaut syndrome; deep brain stimulation; drug-resistant epilepsy; functional neurosurgery; generalized epilepsy; neuromodulation.

FIG. 1.

Reconstruction of bilateral DBS leads in each patient: patient 1 (A), patient 3 (B), patient 4 (C), patient 5 (D), patient 6 (E). Note that patient 2 did not undergo sustained CM-DBS treatment and thus lead positions are not visualized here. For each patient, leads are shown in a sagittal view for the left and right electrodes separately. Locations are displayed with respect to the centromedian (CM), mediodorsal-parafascicular (MD-Pf), and ventral lateral (posterior ventral subdivision) (VLPv) nuclei, as defined by the THOMAS atlas. Bar plots on the right show the spatial overlap between each patient’s bilateral stimulation E-fields and each thalamic nucleus. A = anterior; AV = anterior ventral; Lat. = lateral; P = posterior; Pul = pulvinar; S = superior; VA = ventral anterior; VLa = ventral lateral anterior; VLPd = ventral lateral posterior dorsal; VPL = ventral posterior lateral.

FIG. 2.

Schematic of Boston Scientific DBS lead. Each lead has 4 levels, containing a total of 8 contacts (1 is the deepest contact).

FIG. 3.

Representative scalp EEG recordings obtained in patient 1. A:EEG recording obtained 23 months prior to CM-DBS, showing frequent bursts of SSW lasting up to 30–40 seconds, which were observed during wakefulness and sleep. B: Forty-five-minute EEG recording obtained 1 month after CM-DBS implantation and prior to the DBS device being turned on, showing reduction of SSW during wakefulness (sleep was not captured). C: One-hour EEG recording obtained 6 months after CM-DBS placement, with the device turned on, demonstrating continued reduction of SSW, during wakefulness and sleep.